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A Clinical Studies in Patients with Turner's Syndrome.

Journal of the Korean Pediatric Society 1987;30(10):1143-1151.
Published online October 31, 1987.
A Clinical Studies in Patients with Turner's Syndrome.
Chang Hyun Yang1, Duk Hi Kim1, Kir Young KIm1, Young Ho Yang1
1Department of Pediatrics, College of Medicine, Yonsei University, Seoul, Korea
2Department of Obstetrics and Gynecolgy, College of Medicine, Yonsei University, Seoul, Korea
Turner 증후군에 대 한 임상적 고찰
양창현1, 김덕희1, 김길영1, 양영호1
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 산부인과학교실
Abstract
This study is systematical cytogenetic and clinico-hormonal analysis of 42 cases who were diagnosed as Turner’s syndrome at the Yonsei Medical Center from Jan. 1971 to Dec. 1985. The careful history taking, physical examination, cytogenetic studies, radiologic and hormonal evalua- tion, and GnRH stimulation test were performed. The results were as follows. 1) The patients ranged in age from 14 months to 30 years at the time of diagnosis and median age was 18 years old. 2) Clinically, short stature and sexual infanitilism with primary amenorrhea were found in nearly most cases, except infants and early childhood; with hearing impariment in 5 cases, cardiovascular abonormlities in 5 cases, mental retardation in 4 cases, endocrine abnormalities in 3 cases, and hypoplasia of right kidney in 1 case. 3) The karyotypes showed; ① 45, XO in 19 cases (45.2%) ② 45,XO/46, XX in 13 cases (30.9%) ③ 45, XO/46, XY in 5 cases (11.9%) ④ 45, XO/47, XXX in 2 cases (4.8%) ⑤ 45, XO/46, XX/47, XXX in 1 case (2.4%) ⑥ 45, XO/44, X, -G in 1 case (2.4%) in 1 case (2.4%) ⑦ 46, X, i (Xq) in 1 case (2.4%) 4) The markedly exaggerated rise in both plasma LH and FSH concentration has been demonstrat- ed after pubertal age but plasma Estradiol concentration was normal in prepubertal and low in postubertal compared to normal. 5. 6 cases of Turner’s syndrome patients, who performed GnRH stimulation test, showed that the plasma LH and FSH concentration reached the maximal level at 30 min. after injection of GnRH.
Key Words: Turner’s syndrome


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